Background

The incidence of valvular aortic stenosis (AS) is estimated to be 0.2-0.4 per 1,000 live births.¹ Bicuspid aortic valve (BAV) is the most common cause of AS and one of the most common types of congenital heart disease, with an incidence estimated by autopsy studies of approximately 1%.¹ Dilation of the aorta is a common association with BAV.¹ There is inconsistency in the management of patients with aortic valve disease with a lack of evidence-based guidelines in pediatric patients. Reduction in clinical practice variability in pediatric cardiac patients has been associated with improved resource utilization and patient outcomes.² Thus, a systematic approach to management would be beneficial.

Goals and Details of the Algorithm

The Clinical Practice Algorithms for Aortic Valve Disease were created by the Quality Working Group of the ACC Adult Congenital and Pediatric Cardiology (ACPC) member section. The previously developed secundum atrial septal defect algorithm was used as a precedent in algorithm design.³ The goal was to develop a tool to guide clinical practice in management of pediatric aortic valve disease. The algorithms include recommendations for initial management, outpatient follow-up, and testing frequency, as well as postvalvuloplasty follow-up. The natural history of stenosis and regurgitation was considered along with factors such as age at diagnosis, disease severity, symptoms, and changes in serial imaging.¹

The clinical pathway is designed for management of isolated congenital aortic valve disease. Algorithms were developed for predominant physiology of stenosis or regurgitation. Clinicians should consider the algorithm for the predominant lesion in children with mixed disease. Infants presenting with AS can have more rapid progression of disease resulting in intervention; therefore, closer surveillance is indicated. Predominant aortic regurgitation (AR) is uncommon before intervention in infants, so the regurgitation algorithm is focused on patients >1 year of age.

Excluded diseases include critical AS (often accompanied by more severe left-sided heart disease) and multilevel left ventricular (LV) outflow tract obstruction because the treatment approach differs from isolated valve disease. The hemodynamic demands of pregnancy similarly warrant closer monitoring. An algorithm for adult patients was not created because there are comprehensive pre-existing guidelines for this patient population.⁴ Additionally, genetic or familial aortopathy was not included due to recent pediatric guidelines.⁵

Noninvasive imaging and criteria for grading AS and AR are based on previously published guidelines.⁶ Echocardiographic assessment should be obtained from multiple views with the best Doppler envelope used for measurement. Cardiac MRI (cMRI) and cardiac CT can be useful in assessing aortic dimensions. cMRI can also provide quantitative data on regurgitation, chamber dimensions, hypertrophy, and function. Age and the need for sedation should be considered when determining testing. Cardiopulmonary exercise testing (CPET) or exercise testing (EST) can provide more objective data to assess for ischemic changes and/or cardiac-mediated exercise limitation in age-appropriate patients. CPET or EST may be particularly useful in an asymptomatic individual who does not participate in routine exercise.⁷ Although CPET provides more comprehensive data, EST is acceptable when CPET is not feasible. Abnormalities such as a blunted blood pressure response and ST-segment changes can be evaluated with EST. By CPET, lower peak oxygen consumption and/or blunted oxygen pulse can be useful in determining further management, particularly in AR; however, larger pediatric studies are needed in this area. Although guidance regarding CPET parameters as an indication for intervention is currently lacking, serial trends may be useful.^7,8

Each algorithm begins with an initial clinical evaluation followed by further management based on the degree of stenosis or regurgitation. Indications for intervention include: 1) symptoms such as chest pain, syncope, palpitations, or dyspnea on exertion; and 2) remarkable test results including decrease in ejection fraction (EF) or LV dysfunction (EF <55% or decrease of >10% on serial studies), ST-segment changes on electrocardiogram, and remarkable EST/CPET findings. Mean gradients ≥50 mm Hg by echocardiography regardless of symptoms also warrant intervention. Infants <3 months of age with severe stenosis should be very closely followed due to the high rate of progression (within 1-2 weeks). Parameters used for grading AR in adults are not applicable in smaller children and infants but could be adopted in adolescents close to adult size. In young children, progressive LV enlargement and changes in function along with cardiac symptoms are the main indication for intervention for predominant regurgitant disease.

Although the algorithm provides general recommendations, there may be clinical scenarios that warrant closer follow-up care, and clinician judgment should supplant the algorithm. Shared decision-making is an important consideration; for example, competitive athletes may be considered for earlier intervention after thorough discussion with the patient, family, and primary cardiologist. Additionally, the important association of aortic valve disease with aortopathy may affect evaluation and testing.⁵

The postintervention algorithm guides management and follow-up after initial catheter-based intervention.^9-11 Given the heterogeneity in surgical therapy and postsurgical follow-up, patients who have surgical intervention are excluded in this algorithm.

Methods: Algorithm Development

This algorithm was developed by the Quality Working Group of the ACC ACPC member section. This is a multidisciplinary group comprising pediatric and adult congenital cardiologists with diverse training and practice backgrounds. A quality-driven approach was used to create best practice recommendations as previously modeled.³ Existing guidelines were used when applicable.^4,9,12-14 Group consensus regarding appropriate clinical practice was used to arbitrate conflicts in the literature or when there was a lack of evidence.

Future Directions

The goal of these algorithms is to aid health care professionals in clinical decision-making while caring for pediatric patients with isolated aortic disease. Quality improvement efforts can incorporate this algorithm to improve resource utilization, cost, and patient care in this population.

References

1. Boe BA, Gajarski, RJ. Aortic Stenosis. In: Shaddy RE, Penny DJ, Feltes TF, Cetta F, Mital S, eds. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 10th ed. Vol. 1. Wolters Kluwer; 2021:1049-66.
2. Friedman KG, Rathod RH, Farias M, et al. Resource utilization after introduction of a standardized clinical assessment and management plan. Congenit Heart Dis. 2010;5(4):374-381. doi:10.1111/j.1747-0803.2010.00434.x
3. Plummer ST, Parthiban A, Sachdeva R, Zaidi AN, Statile C. Clinical Practice Algorithm For the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects (ACC website). 2026. Available at: https://www.acc.org/Latest-in-Cardiology/Articles/2022/03/08/19/34/Clinical-Practice-Algorithm-For-the-Follow-up-of-Unrepaired-and-Repaired-SASD. Accessed 09/16/2026.
4. Writing Committee Members, Otto CM, Nishimura RA, et al. 2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2021;77(4):e25-e197. doi:10.1016/j.jacc.2020.11.018
5. Morris SA, Flyer JN, Yetman AT, et al. Cardiovascular management of aortopathy in children: a scientific statement from the American Heart Association. Circulation. 2024;150(11):e228-e254. doi:10.1161/CIR.0000000000001265
6. Zoghbi WA, Adams D, Bonow RO, et al. Recommendations for noninvasive evaluation of native valvular regurgitation: a report from the American Society of Echocardiography developed in collaboration with the Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr. 2017;30(4):303-371. doi:10.1016/j.echo.2017.01.007
7. Cifra B, Cordina RL, Gauthier N, et al. Cardiopulmonary exercise test interpretation across the lifespan in congenital heart disease: a scientific statement from the American Heart Association. J Am Heart Assoc. 2025;14(4):e038200. doi:10.1161/JAHA.124.038200
8. Rhodes J, Fischbach PS, Patel H, Hijazi ZM. Factors affecting the exercise capacity of pediatric patients with aortic regurgitation. Pediatr Cardiol. 2000;21(4):328-333. doi:10.1007/s002460010074
9. Feltes TF, Bacha E, Beekman RH 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation. 2011;123(22):2607-2652. doi:10.1161/CIR.0b013e31821b1f10
10. Materna O, Tax P, Tomek V, et al. Long-term results of congenital aortic stenosis treatment in the era of percutaneous balloon valvuloplasty: up to 33 years follow-up. J Am Heart Assoc. 2023;12(12):e028837. doi:10.1161/JAHA.122.028837
11. Boe BA, Zampi JD, Kennedy KF, et al. Acute success of balloon aortic valvuloplasty in the current era: a National Cardiovascular Data Registry study. JACC Cardiovasc Interv. 2017;10(17):1717-1726. doi:10.1016/j.jcin.2017.08.001
12. Sachdeva R, Valente AM, Armstrong AK, et al. ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 appropriate use criteria for multimodality imaging during the follow-up care of patients with congenital heart disease: a report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography. J Am Coll Cardiol. 2020;75(6):657-703. doi:10.1016/j.jacc.2019.10.002
13. Baumgartner H, Hung J, Bermejo J, et al. Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. J Am Soc Echocardiogr. 2017;30(4):372-392. doi:10.1016/j.echo.2017.02.009
14. Kim JH, Baggish AL, Levine BD, et al. Clinical considerations for competitive sports participation for athletes with cardiovascular abnormalities: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol. 2025;85(10):1059-1108. doi:10.1016/j.jacc.2024.12.025